Three of the four patients had PPNAD; a mother and her daughter with the same PDE11A gene mutation, and a third unrelated patient with a different PDE11A mutation, in which the adrenal glands were described as small (largest total adrenal weight = 6.9 g and normal = 8–9 g) with very minor involvement of the superficial cortex evidenced by a few transcapsular cortical extensions into the peri-adrenal fat (48). The gene discussed is PDE11A; the disease is primary pigmented nodular adrenocortical disease.